I-Richter's Syndrome (RS), eyaziwa nangokuthi inguquko kaRichter, ibhekisela ekuguqulweni kohlobo oluthile lomdlavuza wegazi oluthile oluthile oluhlukumezayo.
I-RS ibhekisela ekuthuthukiseni i- high-grade non-Hodgkin lymphoma kumuntu one- leukemia engalapheki i-lymphocytic (CLL) / encane ye-lymphocytic lymphoma (SLL) . Ezinye izinhlobo ze-RS ziyaziwa nokuthi zenzeka, njengokuguqulwa kwe-Hodgkin lymphoma.
Incazelo yale migomo kanye nokubaluleka kwayo kulandela.
Sibutsetelo
I-RS ivela kumuntu osevele unomdlavuza wamangqamuzana egazi elimhlophe. Lo mdlavuza wokuqala unamagama amabili ahlukene, kuye ngokuthi yikuphi emzimbeni umdlavuza utholakala: Ubizwa nge-CLL uma umdlavuza utholakala kakhulu egazini nasomninini we-bone noma i-SLL uma utholakala ikakhulukazi emagqumeni lymph .
I-CLL isetshenziselwa ukumboza kokubili izinhlangano, ukuqhubeka kulesi sihloko.
Akuwona wonke umuntu onesi-CLL othuthukisa i-Richter Syndrome
Ukuthuthukiswa kwe-RS kubantu abane-CLL akuvamile. Kulinganiselwa ukuthi ukushicilelwa ngo-2016 kungukuthi ukuguqulwa kukaRichter kuvele ngamaphesenti angu-5 kuphela weziguli ezine-CLL. Enye imithombo ichaza ububanzi phakathi kwamaphesenti amabili no-10. Uma i-RS ikwenzeka kuwe, akuyona into engavamile ukuthi kuyokwenzeka ngesikhathi esifanayo i-CLL itholakele. Abantu abakha i-RS kusuka ku-CLL bavame ukwenza kanjalo eminyakeni eminingana emva kokuxilongwa kwe-CLL.
I-Cancer entsha Ngokujwayelekile i-Behaves ngokujulile
Umdlavuza omusha uvela lapho umuntu onesi-CLL eqhubeka nokuthuthukisa lokho okuyaziwa ngokuthi inguquko, kaningi ku-grade-grade engeyona i-Hodgkin lymphoma (NHL). "Ibanga eliphakeme" lisho umdlavuza uvame ukukhula ngokushesha futhi ube nobudlova. I-lymphoma ingumdlavuza wamangqamuzana egazi amhlophe e-lymphocyte.
Ngokwesinye isifundo, amaphesenti angu-90 wezinguquko ukusuka ku-CLL awuhlobo lwe-NHL olubizwa ngokuthi i-B-cell lymphoma enkulu (DLBCL), kanti cishe amaphesenti angu-10 aguqulela eHodgkin lymphoma.
Empeleni ibizwa ngokuthi "i-Hodgkin ehlukile kwe-Richter syndrome (HvRS)" ngalesosikhathi, futhi akucaci ukuthi ukubikezela okuhlukile kuHodgkin lymphoma. Okunye ukuguqulwa okuvela ku-CLL nakho kungenzeka.
Kungani kuthiwa i-Richter Syndrome?
Indoda okuthiwa uMaurice N. Richter okokuqala yachaza lesi sifo ngo-1928. Wabhala ngomabhalane wokuthumelela oneminyaka engu-46 ubudala owangeniswa esibhedlela futhi waba necala eliqhubekayo eliholela ekufeni. Ekuhlaziyweni kwe-autopsy, wanikezela ukuthi bekukhona ukulimala okukodwa okukhona ngaphambili kodwa ukuthi okuvela kuso, ukulimala okusha kubonakala sengathi kuye kwavela okukhula ngokushesha futhi kwagxila futhi kwabhubhisa izicubu ezabe ziyi-CLL endala.
Wabikezela ukuthi i-CLL ikhona isikhathi eside kunanoma ubani owaziyo kulesi siguli, futhi ebhala mayelana namagciwane amabili, noma izilonda, ethi, "Kungenzeka ukuthi ukuthuthukiswa kwesinye sezilonda kwakuxhomeke ekubeni khona komunye . "
Izici
Abantu abane-RS bahlakulela izifo ezinokuhlukumezeka ngokukhula kwe-lymph nodes, ukukhuliswa kwesitebe nesibindi, namazinga aphakanyisiwe omaka egazini elibizwa ngokuthi i-serum lactate dehydrogenase, noma i-LDH.
Isilinganiso sokusinda
Njengawo wonke ama-lymphomas, izibalo zokusinda zingaba nzima ukuzihumusha.
Iziguli ngabanye ziyahlukahluka empilweni yabo nasemandleni abo ngaphambi kokuba zihlolwe. Ukwengeza, ngisho namagciwane amabili anomumo angaziphatha ngendlela ehluke kakhulu kubantu abahlukene. Nokho, nge-RS, umdlavuza omusha unonya kakhulu. Kwamanye abantu abane-RS, kusinda kuye kwabikwa ngesilinganiso samanani ezinyangeni ezingaphansi kwezingu-10 kusukela ekuxilongweni. Kodwa-ke, ezinye izifundo zibonise ukusinda kwezinyanga ezingu-17, kanti abanye abantu abane-RS bangaphila isikhathi eside; I-stem-cell transplantation inganikeza ithuba lokusinda isikhathi eside.
Izimpawu Nezibonakaliso
Uma i-CLL yakho ishintshele ku-DLBCL, uzobona ukuhlukumeza okuphawulekayo kwezimpawu zakho.
Izici ze-RS zihlanganisa ukukhula kwe-tumor ngokushesha noma ngaphandle kokubandakanyeka kwe-extranodal-okungukuthi, ukukhula okusha kungagcinwa kuma-lymph nodes, noma umdlavuza ungabandakanya izitho ngaphandle kwe-lymph nodes, njenge-spleen nesibindi.
Ungase uhlangane:
- Ukwandisa i-lymph nodes ngokushesha
- Ukukhathazeka kwesisu kuhlobene nesipuli esandisiwe nesibindi okuthiwa i-hepatosplenomegaly
- Izimpawu zokubala kwesibalo segazi elibomvu (i-anemia), njengokuzwa ukhathele okwedlulele, isikhumba esilahlekile, ukuphefumula
- Izimpawu zokubala kweplatelet ephansi (thrombocytopenia), njengokulula ukululaza nokungafihli
- Izimpawu zokubandakanyeka kwe- extranodal kufaka phakathi kumasayithi engavamile, njengokwengqondo, isikhumba, isimiso samathumbu, isikhumba namaphaphu
Izingozi Zezinguquko
Ingozi yokuthuthukisa i-RS esuka ku-CLL ayifani nesigaba se-leukemia yakho, isikhathi esingakanani oye wasithola, noma uhlobo lokuphendula ukwelashwa owutholile. Empeleni, ososayensi abaqondi ngokugcwele lokho okubangela ukuguqulwa.
Muva nje, ezinye izifundo ziye zembula ukuthi iziguli ezinama cell CLL ezibonisa uphawu oluthiwa yi-ZAP-70 zingaba nengozi eyengeziwe yokushintsha. Ezinye izimpawu ezinjengeziguli ezine-mutual mutations lapho zixilongwa ziye zaba yenzalo yocwaningo. Noma kunjalo, olunye ucwaningo luye lwasikisela ukuthi iziguli ezincane ze-CLL-okungenani, ezineminyaka engaphansi kwengu-55 ubudala-zingase zibe nengozi eyengeziwe.
Enye imfundiso yukuthi ubude besikhathi eside nesistimu ye-immune ecindezelekile esuka ku-CLL eyenza ushintsho. Kwezinye izinhlobo zeziguli eziye zanciphisa amandla omzimba isikhathi eside, njengokuthi igciwane lesandulela ngculazi (i-HIV) noma kubantu abaye baba nezingxenye zomzimba, kukhona nengozi eyengeziwe yokuthuthukisa i-NHL.
Kungakhathaliseki ukuthi kwenzekani, akubonakali ukuthi kukhona noma yini ongayenza ukuze ubangele noma uvimbele i-CLL yakho ukuba ingaguquki.
Ukwelashwa nokubikezelwa
Ukwelashwa kwe-RS kuvame ukubandakanya izivumelwano ze-chemotherapy ezivame ukusetshenziswa ku-NHL. Lezi zinhlawulo zivame ukukhiqiza izinga lokuphendula ngamaphesenti angama-30. Ngeshwa, ukusinda okuvamile nge-chemotherapy evamile kungaphansi kwezinyanga eziyisithupha ngemuva kokuguqulwa kwe-RS. Izindlela ezintsha zokwelashwa nokuhlanganiswa zilokhu zivivinywa ekuvivinyweni kwemitholampilo, noma kunjalo.
Eminyakeni yamuva, ucwaningo luye lwaphenya ukusetshenziswa kwezinhlelo ze-Fludarabine chemotherapy njengoba kuboniswe ukuthuthukisa imiphumela kwiziguli ezine-CLL eziyinkimbinkimbi . Ukuphila okujwayelekile naloluhlobo lwe-chemotherapy kwanyuka kuya ezinyangeni ezingu-17 kusifundo esisodwa.
Okunye okuqhubekayo kusetshenziswa i -atumumab - isilwane esiphikisayo somuntu olwa nomuntu olwa-anti-CD20 ohlose ithegi ehlukile kuma-lymphocyte B. Ucwaningo lwe- CHOP -O luhlola ukuphepha, ukukwazi, nokusebenza kwe-CHOP chemotherapy ngokuhambisana ne -atumumab ekufakweni nokulondolozwa okulandelayo kweziguli ezine-RS esanda kutholakala. Ngokuhlaziywa okwesikhashana, abangaphezu kuka-7 kwabangu-25 abahlanganyeli bokuqala bafinyelele impendulo ephelele noma eyingxenye emva kwemijikelezo eyisithupha ye-CHOP-O.
Ezinye izifundo ezincane zibuke ukusebenzisa ukusetshenziswa kwe-stem cell transplant ukuphatha lesi sibalo. Iningi lalezi ziguli kulezi zifundo zithole okuningi ngaphambi kwe-chemotherapy. Kuzo izinhlobo ze-stem cell transplants ezivivinyelwe, ukufakelwa okungekho myeloablative kwakunobuthi obuncane, ukufakwa kwe- engraftment engcono, nokuhleleka kokuxolelwa . Kuzofundwa izifundo ezengeziwe ukuze ubone ukuthi lokhu kuyindlela efanelekayo yeziguli ze-RS.
Ucwaningo lwesikhathi esizayo
Ukuze kuthuthukiswe ukusinda kweziguli ezine-RS, ososayensi kudingeka baqonde kangcono ukuthi yini eyenza ushintsho oluvela ku-CLL luzokwenzeka. Ngolwazi oluthe xaxa mayelana ne-RS ezingeni leselula, izindlela zokwelashwa ezingcono zingase zithuthukiswe ngokumelene nalawo ajwayelekile. Kodwa-ke, ochwepheshe baqaphele ukuthi njengoba kunezinguquko eziningana ezinzima ze-molecular ezihlobene ne-RS, kungenzeka ukuthi angeke ibe khona "yokwelashwa okuhlosiwe" okukodwa futhi ukuthi noma iyiphi yale mithi ingase idingeke ihlanganiswe ne-chemotherapy evamile ukuze ithole umphumela omuhle kakhulu. Njengoba ososayensi bevula izimbangela ze-RS, babona ukuthi i-RS ayiyona inqubo efanayo noma inqubo efanayo.
Phakathi naleso sikhathi, iziguli eziye zafaka i-CLL yazo zishintshela ku-RS zikhuthazwa ukuba zibhalise kwizifundo zokwelashwa emzamweni wokuthuthukisa izinketho zokwelapha nemiphumela evela emazingeni amanje.
Imithombo:
Richter, M. Generalized reticular cell sarcoma yama-lymph nodes ahlobene ne-lymphatic leukemia . I-American Journal of Pathology. 1928; 4; 4. 285-292.
Ukuthuthukiswa okusha ku-Richter Syndrome. Eyre TA, uClafford R, uRoberts C, et al. Isandla esisodwa se-NCRI isigaba II se-CHOP ngokuhlanganiswa ne-Ofatumumab ekufakweni nasekugcineni iziguli ezine-syndrome esanda kutholakala. I-BMC Cancer . 2015; 15: 52.
> Parikh SA, Habermann TM, Cha ee KG, et al. Ukuguquguquka kweHodgkin we-chronic lymphocytic leukemia: Isifo, imiphumela, futhi kuqhathaniswa no de novo Hodgkin lymphoma. Am J Hematol. 2015; 90: 334-38.
I-Rossi D, i-Gaidano G. Richter syndrome. I-Adv Exp Med Biol . 2013; 792: 173-91.